title: Sclerema neonatorum treated with intravenous immunoglobulin: a case report and review of treatments.
abstract: Sclerema neonatorum (SN) is a rare neonatal panniculitis that typically develops in severely ill, preterm newborns within the first week of life and often is fatal. It usually occurs in preterm newborns with delivery complications such as respiratory distress or maternal complications such as eclampsia. Few clinical trials have been performed to address potential treatments. Successful treatment has been achieved via exchange transfusion (ET), but its use in neonates is declining. Similar to ET, intravenous immunoglobulin (IVIG) enhances both humoral and cellular immunity and thus may decrease mortality associated with SN. We report a case of SN in a term newborn who subsequently developed septicemia. Biopsy showed subcutaneous, needle-shaped clefts without associated necrosis, inflammation, or calcifications. Treatment with IVIG led to notable but short-term clinical improvement. Sclerema neonatorum remains a poorly understood and difficult to treat neonatal disorder. Although IVIG did not prevent our patient's death, further studies are needed to determine its clinical utility in the treatment of this rare disorder.
authors.affiliation: Department of Dermatology, University of Alabama at Birmingham, 1530 3rd Ave S, EFH Set 414, Birmingham, AL 35294-0009, USA. lchughey@uab.edu.
authors.forename: Kesha J
authors.initials: KJ
authors.lastname: Buster
authors.forename: Holly N
authors.initials: HN
authors.lastname: Burford
authors.forename: Faith A
authors.initials: FA
authors.lastname: Stewart
authors.forename: Klaus
authors.initials: K
authors.lastname: Sellheyer
authors.forename: Lauren C
authors.initials: LC
authors.lastname: Hughey
mesh_terms: D001706:Biopsy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007155:Immunologic Factors / Q000627:therapeutic use*; D007231:Infant, Newborn; D012593:Sclerema Neonatorum / Q000188:drug therapy* / Q000473:pathology; D018805:Sepsis / Q000209:etiology; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors